Munchausen syndrome by proxy and intra-alveolar haemosiderin

Munchausen syndrome by proxy is characterised by the invention of a false history and/or the deliberate inducement of a factitious illness by parents in their child. First reported as a disorder of mothers, this syndrome is now recognised to have male perpetrators. One of the most common characteristic presentations is with the child allegedly suffering repeated apnoeic attacks. These are in fact deliberately induced episodes of upper airway obstruction. The children of these parents are at great risk of serious harm or death if not recognised. The identification of smothering at autopsy is fraught with difficulty and the presence of intra-alveolar haemosiderin has been claimed to be a marker of previous smothering. A case of Munchausen syndrome by proxy is presented where there were deliberate acts of repeated partial smothering and where the finding of intra-alveolar haemosiderin at autopsy provided additional supportive evidence of smothering. Received: 15 June 1998 / Received in revised form: 28 December 1998     

Subtypes and proportions of cerebrovascular disease in an autopsy series in a Japanese Geriatric Hospital

Summary Of 1721 consecutive autopsies performed on patients over 60 years of age in Tokyo Metropolitan Geriatric Hospital, 550 (32% of all autopsied cases) revealed symptomatic cerebrovascular lesions. Among the 550 patients, intracranial hemorrhage was found in 19%, cerebral infarction in 75%, and coexisting cerebral hemorrhage and cerebral infarction in 6%. Twenty-eight percent of the cerebral infarctions were embolic infarctions of cardiac origin, half of which were caused by nonvalvular atrial fibrillation, and 69% were nonembolic infarctions of cardic origin. Progressive subcortical vascular encephalopathy accounted for 15% of the cerebral infarctions. Two-thirds of all lobar cerebral hemorrhages were amyloid angiopathy-related. Nonvalvular atrial fibrillation is the most important cardiac source of embolic stroke. Progressive subcortical vascular encephalopathy is one of the characteristic features of ischemic lesions, and cerebral amyloid angiopathy is an important cause of lobar cerebral hemorrhage in the aged.This work was supported in part by Grant-in-Aid for Cooperative Research No. 63304039, The Ministry of Education, Science and Culture, Japan     

The mysterious death of America’s richest woman in 1917, Mary Lil’ (RIP)

Suspicious circumstances surrounding the death of wealthy socialite Mary Lily “Lil’” Kenan Flagler Bingham in 1917 prompted a secret autopsy conducted by renowned physicians at the request of her relatives. Mrs Bingham, who was formerly married to railroad magnate, Henry Flagler, was among America’s richest people at the time of her death. Mary Lil’s untimely death occurred approximately six months after her marriage to Judge Robert “Bob” Worth Bingham, a college boyfriend whom she married three years after Flagler’s death. In order to establish Mary Lil’s medical condition prior to her death, a book about Mary Lil’s life written by Pulitzer Prize author, David Chandler and articles from the New York Times; The Louisville Courier Journal, a Kentucky newspaper; and Mary Lil’s hometown newspaper, The Morning Star, in Wilmington, North Carolina were examined. Although a team of medical experts performed a secret autopsy following her death and released their findings to her family, the examination and tests results remain undisclosed to the public.     

Managed care and measuring medical outcomes: Did the rise of HMOs contribute to the fall in the autopsy rate?

The U.S. autopsy rate has fallen precipitously since the 1940s, decreasing from 50 percent of bodies to less than eight percent today. Much of the decrease occurred after 1971 when hospitals were no longer required to do a minimum number of autopsies for accreditation. Since this time, major changes in the health care sector have occurred in the United States, highlighted by the increased importance of managed care. Using data for 46 states from 1987 to 2000, we analyze the degree to which the rise in manage care explains the decrease in the autopsy rate. We find that increases in health maintenance organization market share explain 21 percent of the decrease in the autopsy rate over the years from 1987 to 2000 and reductions in the number of hospital deaths explain another 30 percent. In contrast, we find that increases in the availability of magnetic resonance imaging had no significant effect on autopsy rates when other factors are held constant. Reforming health care financing to restrain the growth in health care costs using incentive mechanisms similar to those employed by managed care organizations has been a recurring policy goal in the United States. Our results imply that these reforms may inadvertently reduce the incentive to monitor medical outcomes using techniques such as autopsies, which is often called the “gold standard” in measuring medical outcomes.     

全身性韦格内肉芽肿病─附2例尸检研究

对２例全身性韦格内肉芽肿病的尸检病例进行了研究。发现上、下呼吸道有坏死性肉芽肿和小血管炎，肾脏有局灶节段坏死性肾小球肾炎。病变还累及消化道、皮肤、肝、脾、心脏、膀胱、肾上腺和淋巴结。本文就韦格内肉芽肿病和淋巴瘤样肉芽肿的鉴别诊断进行了讨论。     

原发性免疫缺陷病尸检1例形态学观察及文献复习

目的　探讨原发性免疫缺陷病各脏器的病理组织特征及免疫器官在不同免疫状态下的形态、功能改变。方法　总结 1例原发性免疫缺陷病患儿的临床资料和尸检病理学特点 ,用抗酸、PAS、六胺银等特殊染色及AE1/AE3、CD1a、CD3、CD2 0、CD10、CD6 8、CD2 1、S 10 0、plasmacell、CD34等免疫组化染色协助诊断。结果　患儿胸腺、淋巴结、脾淋巴组织发育不良 ,T、B淋巴细胞数目都明显减少。特异性联合免疫缺陷导致肺脓肿 ,肝、脾、淋巴结结核 ,全身多脏器巨细胞病毒感染 ,肺卡氏肺囊虫等广泛、难以纠正的感染。结论　免疫系统成分多样 ,功能复杂 ,在机体不同免疫状态及外界不断刺激下 ,其形态学改变复杂。对于免疫系统疾病 ,需结合临床资料、形态学观察及必需的分子生物学技术进行诊断和鉴别诊断。     

肝原发性神经内分泌癌1例尸检报道并文献复习

目的探讨肝原发性神经内分泌癌的病理组织学、免疫组织化学以及超微结构特征。方法对1例尸检诊断的肝原发性神经内分泌癌病理组织学、免疫组织化学以及超微结构进行研究,并结合文献复习。结果男性,84岁。肝重2 410 g,可见大小不等的结节弥漫分布,最大者5 cm×4 cm×4 cm。瘤细胞小至中等大小,呈弥漫分布的巢状结构,富于血窦。瘤细胞免疫表型AE1、Syn、CD99、NSE和VIP(+),LCA、AFP、Hepar-1(-)。电镜下可见成簇的大小为150~350 nm不等的电子密度致密的核心颗粒。结论肝原发性神经内分泌癌非常少见,其诊断需结合病理组织形态、免疫表型及超微结构特征,并除外其他脏器原发病变的肝转移。     

Effect of formalin fixation on pcr amplification of DNA isolated from healthy autopsy tissues

The aim of this study is to investigate the effects of formalin fixation on the degradation of DNA molecules in five different healthy tissues exempted during the autopsy, as well as the selection of the method that is most suitable for the DNA isolation. Heart muscle, liver, brain, lung and kidney tissue obtained from the healthy people who suddenly died from a violent death were used. The parts of tissue were fixed in 10% phosphate-buffered formalin as well as in 4% unbuffered formalin at room temperature. Morphology of tissue was studied using H&E staining. The DNA was isolated 6?h, 1–7 days (every 24?h), 10, 14, 28 days and 2 months after fixation using two different methods: extraction with phenol-chloroform-isoamyl alcohol as well as with PureLink Genomic DNA Kit. Yield and purity of the DNA samples were measured spectrophotometrically at 260?nm and 280?nm. The PCR amplifications of the glycerol-3-phosphate dehydrogenase 1 (GPD1, 150 bp), ß actin (ACTB, 262 bp) and ribosomal protein L4 (RPL4, 407 bp) genes were performed to evaluate the degree of DNA fragmentation. The RPL4 gene was amplified up to 72?h, ACTB gene up to 14 days and GPD1 gene up to 28 days from tissue fixed in phosphate-buffered formalin using phenol-chloroform-isoamylalcohol protocol for DNA isolation. Liver and kidney gave better results of PCR amplification, but statistical significance between tissues was not found. Preserving period, fixative and DNA extracting method are important factors for successful PCR amplification. The healthy tissue, fixed in phosphate-formalin up to 28 days, can be useful source in molecular studies. Tissues fixed in unbuffered formalin are suitable for molecular analysis up to 7 days.     

Utility of Autopsy among Pediatric Allogeneic Hematopoietic Stem Cell Transplant Recipients: One Last Chance to Learn?

Autopsy may confirm clinical diagnoses or identify conditions that were not suspected prior to a patient's death. Previous studies evaluating the utility of autopsy in hematopoietic stem cell transplant (HSCT) recipients yielded conflicting results.We conducted a retrospective cohort study of children (<18 years of age) undergoing allogeneic HSCT at Duke University who died of any cause between January 1, 1995, and December 31, 2016. We evaluated associations between patient characteristics and autopsy performance using chi-square or Fisher exact tests. We reviewed autopsy reports to determine the concordance between preautopsy causes of death and pathological diagnoses identified on autopsy. We classified unexpected diagnoses on autopsy using criteria developed by Goldman et al. We evaluated for temporal changes in the autopsy consent rate and the frequency of unexpected diagnoses on autopsy using Cochran-Armitage tests.During the 22-year study period, 475 patients died and had data available on autopsy performance, and 130 (27%) of these patients underwent autopsy. The autopsy consent rate declined over time (P < .0001), with autopsies being performed for 40% of deaths in 1995 to 1999 and 17% of deaths in 2009 to 2016. White patients were more likely to undergo autopsy than nonwhite patients (P?=?.03). There were no associations between autopsy performance and patient age, sex, HSCT indication, or HSCT donor. Unexpected diagnoses were identified in 31 (24%) autopsies. The proportion of autopsies with an unexpected diagnosis did not change during the study period (P?=?.45). However, infectious diagnoses that would have led to a change in management were more frequently identified on autopsies in 1995 to 2003 than in 2004 to 2016 (20% versus 0%; P?=?.001).The autopsy consent rate for pediatric HSCT recipients at our institution has declined substantially over the past several decades. The utility of autopsy in this patient population remains high despite a reduction in the identification of unexpected infections.     

117例先天性心血管畸形尸检的临床病理学研究

目的:分析117例先天性心血管畸形尸检的临床病理资料,以提高其诊断水平。方法:收集我院1984年至2009年414例尸检标本中的117例先天性心血管畸形尸检材料,应用心脏节段分析法并结合临床资料进行分析。结果:1.先天性心血管畸形尸检的检出率为28.3%(117/414例),其中男性70例,女性47例,年龄孕18周～34岁。2.先天性心血管畸形的病变类型为:大静脉病变13例、心房病变11例、房室连接病变5例、心室病变51例、心室动脉连接病变27例及大动脉病变10例。其中室间隔缺损23例,检出率为19.7%(23/117),法洛四联症19例,检出率为16.2%(19/117)。3.心外伴发畸形中常见脾脏畸形9例、多指(趾)畸形3例及肺叶畸形5例等。4.先天性心血管畸形的术后死亡原因主要有低心排出量综合征(低心排)、肺淤血水肿、感染出血、弥散性血管内凝血及肺透明膜形成等。结论:心脏节段分析方法能更好对先天性心血管畸形进行分析和命名;尸检是确定死因的重要手段,通过尸检为进一步拓宽临床诊断和手术治疗提供病理学基础。